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Primary cilia

Kelsey H Elliott, Samantha A Brugmann
Molecular signals are the guiding force of development, imparting direction upon cells to divide, migrate, differentiate, etc. The mechanisms by which a cell can receive and transduce these signals into measurable actions remains a 'black box' in developmental biology. Primary cilia are ubiquitous, microtubule-based organelles that dynamically extend from a cell to receive and process molecular and mechanical signaling cues. In the last decade, this organelle has become increasingly intriguing to the research community due to its ability to act as a cellular antenna, receive and transduce molecular stimuli, and initiate a cellular response...
March 13, 2018: Developmental Biology
Kurt A Zimmerman, Cheng Jack Song, Nancy Gonzalez-Mize, Zhang Li, Bradley K Yoder
Hepatorenal fibrocystic disease (HRFCD) is characterized by cysts in the kidney and liver with associated fibrosis and is the result of defects in proteins required for cilia function or assembly. Previous reports indicate that macrophages, mainly M2-like macrophages, contribute to HRFCD, although the origin (yolk-sac derived resident macrophages vs bone-marrow derived infiltrating macrophages) and contribution of these cells to the observed phenotypes is unknown. Herein, we utilize a congenital model of cilia dysfunction (IFT88Orpk ) to study the importance of macrophages in HRFCD...
March 15, 2018: American Journal of Physiology. Gastrointestinal and Liver Physiology
Peixuan Xia, Shihua Chu, Geng Liu, Guoqing Chen, Tao Yi, Shi Feng, Hongying Zhou
Kinesin Family Member 3A (KIF3A) was recognized as a key factor of ciliogenesis and transport system of primary cilia in normal cells. However, its possible function on cancer cells has yet to be identified. In the present study, microarray tissue chips, including 230 breast cancer samples, were applied to determine the KIF3A expression pattern by immunological histological chemistry. Statistical analysis on the KIF3A expression level and the currently used clinicopathological characteristics of breast cancer patients was carried out...
April 2018: Biomedical Reports
Dhivya Kumar, Rebecca T Thomason, Maya Yankova, Jonathan D Gitlin, Richard E Mains, Betty A Eipper, Stephen M King
The assembly of membranous extensions such as microvilli and cilia in polarized cells is a tightly regulated, yet poorly understood, process. Peptidylglycine α-amidating monooxygenase (PAM), a membrane enzyme essential for the synthesis of amidated bioactive peptides, was recently identified in motile and non-motile (primary) cilia and has an essential role in ciliogenesis in Chlamydomonas, Schmidtea and mouse. In mammalian cells, changes in PAM levels alter secretion and organization of the actin cytoskeleton...
March 14, 2018: Scientific Reports
Thibaut Eguether, Fabrice P Cordelieres, Gregory J Pazour
The vertebrate hedgehog pathway is organized in primary cilia and hedgehog components relocate into or out of cilia during signaling. Defects in intraflagellar transport (IFT) typically disrupt ciliary assembly and attenuate hedgehog signaling. Determining if IFT drives the movement of hedgehog components is difficult due to the requirement of IFT for building cilia. Unlike most IFT proteins, IFT27 is dispensable for cilia formation but affects hedgehog signaling similar to other IFTs allowing us to examine its role in the dynamics of signaling...
March 14, 2018: Molecular Biology of the Cell
Kirti K Chahal, Milind Parle, Ruben Abagyan
The hedgehog (Hh) pathway plays an important role in cancer development and maintenance, as ~25% of all cancers have aberrant Hh pathway activation. Targeted therapy for inhibition of the Hh pathway was thought to be promising for achieving clinical response in the Hh-dependent cancers. However, the results of new clinical trials with smoothened (SMO) antagonists do not show much success in cancers other than basal cell carcinoma. The studies suggest that the Hh pathway involves multiple mechanisms of activation or inhibition in primary cilia and interactions between several related pathways in different types of cells, which makes this pathway extremely complex...
March 13, 2018: Anti-cancer Drugs
Kelsey H Elliott, Grethel Millington, Samantha A Brugmann
BACKGROUND: Submandibular glands (SMGs) are specialized epithelial structures which generate saliva necessary for mastication and digestion. Loss of SMGs can lead to inflammation, oral lesions, fungal infections, problems with chewing/swallowing, and tooth decay. Understanding the development of the SMG is important for developing therapeutic options for patients with impaired SMG function. Recent studies have suggested Sonic hedgehog (Shh) signaling in the epithelium plays an integral role in SMG development; however, the mechanism by which Shh influences gland development remains nebulous...
March 13, 2018: Developmental Dynamics: An Official Publication of the American Association of Anatomists
Yi Xu, Ho Jung Ju, S DeBlasio, Elizabeth J Carino, R Johnson, M MacCoss, M Cilia Heck, W Allen Miller, S M Gray
Translational readthrough of the stop codon of the capsid protein (CP) open reading frame (ORF) is used by members of the Luteoviridae to produce their minor capsid protein as a readthrough protein (RTP). The elements regulating RTP expression are not well understood, but involve long-distance interactions between RNA domains. Using high-resolution mass spectrometry, glutamine and tyrosine were identified as the primary amino acids inserted at the stop codon of Potato leafroll virus (PLRV) CP ORF. We characterized the contributions of a cytidine-rich domain immediately downstream, and a branched stem-loop structure 600-700 nucleotides downstream of the CP stop codon...
March 7, 2018: Journal of Virology
Maren Mönnich, Louise Borgeskov, Loretta Breslin, Lis Jakobsen, Michaela Rogowski, Canan Doganli, Jacob M Schrøder, Johanne B Mogensen, Louise Blinkenkjær, Lea M Harder, Emma Lundberg, Stefan Geimer, Søren T Christensen, Jens S Andersen, Lars A Larsen, Lotte B Pedersen
The centrosome is the main microtubule-organizing center in animal cells and comprises a mother and daughter centriole surrounded by pericentriolar material. During formation of primary cilia, the mother centriole transforms into a basal body that templates the ciliary axoneme. Ciliogenesis depends on mother centriole-specific distal appendages, whereas the role of subdistal appendages in ciliary function is unclear. Here, we identify CEP128 as a centriole subdistal appendage protein required for regulating ciliary signaling...
March 6, 2018: Cell Reports
Lv Liu, Hong Luo
Primary ciliary dyskinesia (PCD) is a clinical rare peculiar disorder, mainly featured by respiratory infection, tympanitis, nasosinusitis, and male infertility. Previous study demonstrated it is an autosomal recessive disease and by 2017 almost 40 pathologic genes have been identified. Among them are the leucine-rich repeat- (LRR-) containing 6 (LRRC6) codes for a 463-amino-acid cytoplasmic protein, expressed distinctively in motile cilia cells, including the testis cells and the respiratory epithelial cells...
2018: BioMed Research International
Federico Gnesotto, Federica Mura, Jannes Gladrow, Chase Broedersz
Living systems operate far from thermodynamic equilibrium. Enzymatic activity can induce broken detailed balance at the molecular scale. This molecular scale breaking of detailed balance is crucial to achieve biological functions such as high-fidelity transcription and translation, sensing, adaptation, biochemical patterning, and force generation. While biological systems such as motor enzymes violate detailed balance at the molecular scale, it remains unclear how non-equilibrium dynamics manifests at the mesoscale in systems that are driven through the collective activity of many motors...
March 5, 2018: Reports on Progress in Physics
Anne-Clémence Vion, Silvanus Alt, Alexandra Klaus-Bergmann, Anna Szymborska, Tuyu Zheng, Tijana Perovic, Adel Hammoutene, Marta Bastos Oliveira, Eireen Bartels-Klein, Irene Hollfinger, Pierre-Emmanuel Rautou, Miguel O Bernabeu, Holger Gerhardt
Blood flow shapes vascular networks by orchestrating endothelial cell behavior and function. How endothelial cells read and interpret flow-derived signals is poorly understood. Here, we show that endothelial cells in the developing mouse retina form and use luminal primary cilia to stabilize vessel connections selectively in parts of the remodeling vascular plexus experiencing low and intermediate shear stress. Inducible genetic deletion of the essential cilia component intraflagellar transport protein 88 (IFT88) in endothelial cells caused premature and random vessel regression without affecting proliferation, cell cycle progression, or apoptosis...
March 2, 2018: Journal of Cell Biology
Jacelyn Emily Peabody, Ren-Jay Shei, Brent M Bermingham, Scott Edward Phillips, Brett Turner, Steven M Rowe, George Martin Solomon
The respiratory tract is lined with multi-ciliated epithelial cells that function to move mucus and trapped particles via the mucociliary transport apparatus. Genetic and acquired ciliopathies result in diminished mucociliary clearance, contributing to disease pathogenesis. Recent innovations in imaging technology has advanced our understanding of ciliary motion in health and disease states. Application of imaging modalities including transmission electron microscopy (TEM), high-speed video microscopy (HSVM), and micron-optical coherence tomography (μOCT) could improve diagnostics and be applied for precision medicine...
March 1, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
Michele A Corrigan, Gillian P Johnson, Elena Stavenschi, Mathieu Riffault, Marie-Noelle Labour, David A Hoey
Skeletal homeostasis requires the continued replenishment of the bone forming osteoblast from a mesenchymal stem cell (MSC) population, a process that has been shown to be mechanically regulated. However, the mechanisms by which a biophysical stimulus can induce a change in biochemical signaling, mechanotransduction, is poorly understood. As a precursor to loading-induced bone formation, deciphering the molecular mechanisms of MSC osteogenesis is a critical step in developing novel anabolic therapies. Therefore, in this study we characterize the expression of the mechanosensitive calcium channel Transient Receptor Potential subfamily V member 4 (TRPV4) in MSCs and demonstrate that TRPV4 localizes to areas of high strain, specifically the primary cilium...
February 28, 2018: Scientific Reports
Ae Lee Jeong, Hye In Ka, Sora Han, Sunyi Lee, Eun-Woo Lee, Su Jung Soh, Hyun Jeong Joo, Buyanravjkh Sumiyasuren, Ji Young Park, Jong-Seok Lim, Jong Hoon Park, Myung Sok Lee, Young Yang
In most mammalian cells, the primary cilium is a microtubule-enriched protrusion of the plasma membrane and acts as a key coordinator of signaling pathways during development and tissue homeostasis. The primary cilium is generated from the basal body, and cancerous inhibitor of protein phosphatase 2A (CIP2A), the overexpression of which stabilizes c-MYC to support the malignant growth of tumor cells, is localized in the centrosome. Here, we show that CIP2A overexpression induces primary cilia disassembly through the activation of Aurora A kinase, and CIP2A depletion increases ciliated cells and cilia length in retinal pigment epithelium (RPE1) cells...
February 28, 2018: EMBO Reports
Massimo Pifferi, Andrew Bush, Michele Rizzo, Alessandro Tonacci, Maria Di Cicco, Martina Piras, Fabrizio Maggi, Giulia Paiola, Angela Michelucci, Angela Cangiotti, Diego Peroni, Davide Caramella, Attilio L Boner
Cilia have multiple functions including olfaction. We hypothesised that olfactory function could be impaired in primary ciliary dyskinesia (PCD). Olfaction, nasal nitric oxide (nNO) and sinus CT were assessed in patients with PCD and non-PCD sinus disease, and healthy controls (no CT scan). PCD and non-PCD patients had similar severity of sinus disease. Despite this, defective olfaction was more common in patients with PCD (P<0.0001) and more severe in patients with PCD with major Transmission Electron Microscopy (TEM) abnormalities...
February 28, 2018: Thorax
Elizabeth Forsythe, Joanna Kenny, Chiara Bacchelli, Philip L Beales
Bardet-Biedl syndrome is a rare autosomal recessive multisystem disorder caused by defects in genes encoding for proteins that localize to the primary cilium/basal body complex. Twenty-one disease-causing genes have been identified to date. It is one of the most well-studied conditions in the family of diseases caused by defective cilia collectively known as ciliopathies. In this review, we provide an update on diagnostic developments, clinical features, and progress in the management of Bardet-Biedl syndrome...
2018: Frontiers in Pediatrics
Chengbing Wang, Jia Li, Ken-Ichi Takemaru, Xiaogang Jiang, Guoqiang Xu, Baolin Wang
The primary cilium is a microtubule-based organelle required for Hedgehog (Hh) signaling and consists of basal body, ciliary axoneme, and transition zone, a compartment between the first two structures. The transition zone serves as a gatekeeper to control protein composition in cilia, but less is known about its role in ciliary bud formation. Here, we show that centrosomal protein Dzip1l is required for Hh signaling between Smoothened and Sufu. Dzip1l colocalizes with basal body appendage proteins and Rpgrip1l, a transition zone protein...
February 27, 2018: Development
Li Eon Kuek, Paul Griffin, Paul Martinello, Alison N Graham, Paul Kalitsis, Philip J Robinson, Graham A Mackay
Primary ciliary dyskinesia (PCD) is an inherited, currently incurable condition. In the respiratory system, PCD causes impaired functioning of the mucociliary escalator leading to nasal congestion, cough and recurrent otitis media which commonly progresses to cause more serious and permanent damage including hearing deficits, chronic sinusitis and bronchiectasis. New treatment options for the condition are thus necessary. In characterising an immortalised human bronchial epithelial cell line (BCi-NS1.1), grown at an air/liquid interface to permit differentiation, we have identified that these cells have dyskinetic motile cilia...
February 26, 2018: American Journal of Respiratory Cell and Molecular Biology
Junguee Lee, Dong Hyun Oh, Ki Cheol Park, Ji Eun Choi, Jong Beom Kwon, Jongho Lee, Kuhn Park, Hae Joung Sul
Primary cilia are solitary, non-motile, axonemal microtubule-based antenna-like organelles that project from the plasma membrane of most mammalian cells and are implicated in transducing hedgehog signals during development. It was recently proposed that aberrant SHH signaling may be implicated in the progression of idiopathic pulmonary fibrosis (IPF). However, the distribution and role of primary cilia in IPF remains unclear. Here, we clearly observed the primary cilia in alveolar epithelial cells, fibroblasts, and endothelial cells of human normal lung tissue...
February 23, 2018: Molecules and Cells
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